Antiphospholipid Antibody Syndrome (APLS; APS)

“Antiphospholipid Antibody Syndrome” is an immune disorder characterized by a hyper coagulable state meaning it is a condition characterised by tendency to develop clots in medium to large arteries or large veins.

As a result the patient has a tendency towards development of clots in any organ of the body. A few examples of these clots leading to complications are given below:

  1. Large veins causing a condition called DVT (Deep Vein Thrombosis).
  2. Blood clots in large veins may break off and get lodged in the arteries of the lungs causing a life threatening condition called “Pulmonary Embolism”.
  3. Similarly clots in the arteries of the heart can lead to heart attacks, or in the brain giving rise to strokes, or even intestines causing intestinal gangrene etc.
  4. Compromised arterial supply to the  placenta during pregnancy , very often causes recurrent miscarriages. Sometimes there may not be history of a miscarriage but the patient may have pre-pre-eclampsia during pregnancy which is a condition characterized by very high blood pressure and loss of protein in the Urine.

 

The examples given above are few of the major ones however the fact of the matter is that APLS can cause clots in any organ of the body including micro thrombi (small Thrombi) in the skin causing skin ulcers or the retinal arteries causing Retinal Thrombosis etc.

Other features of this disease include reduced platelet counts or changes in the appearance of the skin which shows a mottled appearance and is called Livedoreticularis.

APLS is quite often found to occur in patients of connective tissue diseases such as SLE but can also occur as an independent illness in which case it is known as Primary APLS.

Diagnosis of Antiphospholipid Antibody Syndrome:

There is a set of blood tests which detect antibodies responsible for causing APLS. These antibodies act by binding to phospholipids which are important components in the blood clotting mechanisms. It is for this reason that the syndrome is called Antiphospholipid Antibody syndrome (APLS). The laboratory tests are:

  1. Anti-cardiolipin Antibody test
  2. Beta 2 Glycoprotein 1
  3. Lupus Anticoagulant

Treatment of Antiphospholipid Antibody Syndrome:

The available treatment options for management of APLS comprise of anticoagulants and anti platelet drugs. Once an episode of clotting has occurred and diagnosed to have occurred secondary to APLS, treatment is lifelong for all intents and purposes. There are no guidelines on if and when the treatment can be stopped. The treatment of APLS comprises of either Heparin injections or oral anticoagulant medications available as tablets which are called Coumadin.

Heparin injections are of two types the conventional heparin and low molecular weight heparin. The low molecular weight heparin is after and monitoring of blood tests to assess the degree of blood thinning is not required. However these injections are a little expensive for regular long term use as happens during pregnancy state.

Oral Coumadin is available in tablet form and requires regular monitoring of the thickness of the blood by means of a blood test called the INR which has to be done very frequently initially and a little less later on. This is because excessive thinning of blood can give rise to complications of bleeding from multiple site such as the gums, urine, excessive bleeding during menses etc. On the other hand inadequate anticoagulation can give rise to future attacks of Clotting episodes.

There are dietary restrictions when patients are on oral Coumadin. Patients are to avoid Vitamin K rich foods else the effect of Coumadin is reduced. There are additional guidelines to be followed which are given separately under the heading of “Instructions for patients on Anticoagulants”.

Coumadin cannot be given during pregnancy and patients desirous of pregnancy will need to be on heparin injections throughout the pregnancy and for 6 weeks after delivery.
The heparin needs to be started from the moment the pregnancy test is positive.

Steroids and Immunosuppressants have been tried in this illness as well however results have been variable and hence as of now there are no concrete guidelines on the use of these drugs in APLS.